Comparative study of validity of clinical, X‐ray and magnetic resonance imaging scores in evaluation and management of haemophilic arthropathy in children

Abstract

To evaluate joints alterations, we performed clinical examination, X-rays and magnetic resonance imaging (MRI) (Denver score) in 165 joints of 40 children with severe (n, 32) or moderate (n, 8) haemophilia A or B. All investigated joints had a history of more than three bleeds. At evaluation, 25 of 40 haemophilic patients were on prophylaxis for the last 1-8 years (mean: 3.5 years). MRI revealed chronic synovitis in 55.4% and 50% of joints, which were diagnosed, as normal by the clinical scale and plain radiography respectively. Moreover, MRI unmasked more profound alterations than those observed by plain radiography in 70% of cases. Statistical analysis showed that the clinical and Pettersson scores in contrast to the Denver score provide an underestimation of arthritic changes. Besides, Denver score did not provide resolution in differentiating stages of arthropathy, because of its inherent nature; however, a score of 6 expressing severe synovitis seemed to be the cut-off value for the distinction of severe cases. Based on MRI findings we intensified prophylaxis in nine children and initiated it in another nine children. Five children, who were already on prophylaxis complied with our recommendations and eliminated haemorrhages. Finally, three boys with severe haemophilic arthropathy in knees underwent successful chemical synovectomy with rifampicin.

Safety and pharmacokinetics of a novel recombinant fusion protein linking coagulation factor IX with albumin (rIX-FP) in hemophilia B patients

“…1 Recurrent hemarthroses result in progressive joint damage and the development of painful and debilitating hemophilic arthropathy. 2 Replacement therapy with FIX products, manufactured from plasma (pdFIX) or more recently by recombinant technology (rFIX), is presently used to control and prevent bleeding episodes. 3,4 Prophylaxis is currently considered optimal care for patients with severe hemophilia 5 because it reduces the incidence of all bleeding episodes and, when initiated at a very young age, also reduces the risk of arthropathy development.…”

MRI demonstrates the extension of juxta-articular venous malformation of the knee and correlates with joint changes

“…Second, joint destruction from inflammation due to recurrent articular haemorrhage. The latter mechanism appears similar to the changes seen in haemophilic arthropathy, with synovial thickening and haemosiderin deposits initiating the inflammatory changes leading to arthropathy [10,11].…”

A Comparison of Efficacy Between Recombinant Activated Factor VII (Aryoseven) and Novoseven in Patients With Hereditary FVIII Deficiency With Inhibitor

“…We checked mean levels of FVII before and after administration of the 2 rFVIIa preparations to prove FVII: C level was comparably increased. 6,38 In the previous studies, the rate of reported adverse events was 1% to 10%, [50][51][52][53][54][55][56][57][58][59][60] and the most of adverse events had been headache, rashes, hypertension, and rarely thrombosis. We did not encounter any thrombosis in either treatment groups.…”